Estimates of the prevalence of nervous system involvement in SLE patients range from 14% to over 90%, depending on severity and type of symptoms considered (1,2). Patients can present with seizures, stroke, and altered mental status including coma, psychosis, dementia, and chorea. Transverse myelitis, peripheral neuropathy, and myositis can also occur. Less focal manifestations can include headaches, paresthesias, anxiety states, and cognitive difficulties. NPSLE syndromes have recently been classified by the American College of Rheumatology (3). Treatment can further confound the diagnostic challenge, since drugs may lead to neuropsychiatric side effects, such as psychosis in high-dose steroid treatment or secondary infectious or neoplastic complications in the immunocompromised patient. Difficult questions arise: should the patient receive more aggressive therapy or less?

Acute Injury in SLE: Infarct

Antiphospholipid antibody is common in SLE.Thrombosis and infarct occur in the brain as well as elsewhere in the body in patients with antiphospholipid antibody.Sudden onset of neurological change in a patient with SLE should suggest the possibility of infarct.By the same token, the diagnosis of cerebral infarct in a relatively young patient should suggest the possibility of antiphospholipid antibody and SLE.Infarcts can range in size from large vessel territories to small, lacunar infarcts.

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Figure 1. Acute infarct. A 23-year-old woman with SLE had sudden onset of aphasia. Diffusion-weighted MR image (A) shows acute left frontal lobe infarct. The cortical signal abnormality is much more subtle on the FLAIR image (B). Spectroscopy with a voxel placed over the abnormal diffusion lesion using PRESS technique, TE 35 msec (C) shows prominent lactate doublet (white arrow) and decreased NAA peak (yellow arrow). The patient had antiphospholipid antibody.

Figure 2. Acute infarct. Photomicrograph from autopsy of a different patient with SLE and antiphospholipid antibody shows edge of cortex (lower half) and thrombus in a small vessel in the subarachnoid space (arrow) (H & E stain).